Rituximab in childhood lupus myocarditis

Abstract

Sir, Myocarditis is a life-threatening complication of systemic lupus erythematosus (SLE) [1]. Myocardial involvement demonstrable as reduction in left ventricular ejection fraction can be seen in 8–25% of patients with active SLE [2]. Symptomatic myocarditis, however, with signs of frank congestive heart failure is extremely uncommon especially in childhood lupus [3, 4]. We report a young girl with lupus myocarditis, who had severe congestive cardiac failure while on maximal immunosuppression. R, a 12-year-old girl, presented with complaints of polyarticular arthritis, fever, edema, and painless oral ulcers. She had pallor, pitting pedal edema, hypertension, and arthritis involving knee, ankle, and proximal interphalangeal joints. On investigation, urine examination revealed many red blood cells, and 24-h urine protein was 136 mg/m/h. Blood urea was 56 mg/dl and serum creatinine 1.4 mg/dl. Antinuclear antibody was 4+, diVuse pattern and antidsDNA antibody titer was 760 IU/ml (N < 55). Lupus anticoagulant was positive. Renal biopsy revealed class IV/V lupus nephritis. She was administered Wve daily methylprednisolone pulses (30 mg/kg/dose) followed by oral prednisolone (2 mg/kg/ day). She was also started on intravenous monthly cyclophosphamide pulses (750 mg/m/dose). Within a month, she was readmitted with fever, increasing edema, and respiratory distress. She had persistent disease activity, nonoliguric renal failure (creatinine 3.4 mg/dl), and hypertension. She was treated again with intravenous pulse methylprednisolone, and cyclophosphamide pulses were continued. With above treatment, renal functions normalized and blood pressure got controlled with antihypertensive medications—amlodipine (0.3 mg/kg/day) and carvedilol (0.6 mg/kg/day). Three months later, she presented with loose stools, vomiting for 2 days, and respiratory distress for a day. On examination, she had tachycardia, tachypnoea, and hypotension. She developed basal crepts, hepatomegaly, and gallop rhythm. For hemodynamic stability, she required inotropic support. Chest X-ray showed cardiomegaly (cardio thoracic ratio 64%) and bilateral basal inWltrates. Ventilation/perfusion (V/Q) scan was normal. Echocardiography showed dilated right and left ventricles, decreased myocardial contractility with left ventricular ejection fraction of only 30%. A clinical diagnosis of lupus myocarditis was made. As she had already received two courses of intravenous methylprednisolone and was on monthly cyclophosphamide pulses, a decision was made to start her on rituximab (375 mg/m/dose). She showed prompt clinical recovery, and we were able to taper oV her inotropes. The gallop disappeared. Repeat echocardiogram done after 1 week showed ejection fraction of 50%. She was treated with weekly doses of rituximab (375 mg/m/dose) for 4 weeks. Cyclophosphamide was given every month for 6 months and thereafter every 3 months. After 12 months of follow-up patient, she is asymptomatic and on tapering doses of oral prednisolone along with hydroxychloroquine. Cardiac involvement represents one of the most important clinical manifestations of SLE and contributes signiWcantly to the morbidity and P. Aggarwal · S. Singh (&) · D. Suri · A. Rawat Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India e-mail: surjitsinghpgi@rediffmail.com